Oral Juvenile Xanthogranuloma: A Clinicopathological, Immunohistochemical and BRAF V600E Study of Five New Cases, with Literature Review

Mota, Carolina Peres; Cunha, John Lennon Silva; Magalhães, Maria Carolina Silva Versieux; Israel, Mônica Simões; Freire, Nathália De Almeida; Santos, Emília Rosaria Carvalho Dos; Canêdo, Nathalie Henriques Silva; Agostini, Michelle; Abrahão, Aline Corrêa; Andrade, Bruno Augusto Benevenuto de; Romañach, Mário José; Sousa, Sílvia Ferreira de

doi:10.1007/s12105-021-01373-x

Cited by 6 publications

(9 citation statements)

References 36 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…JXG is a histiocytic disorder with frequent cutaneous involvement. [14][15][16] Microscopically, JXG exhibits a proliferation of histiocytes with a foamy, eosinophilic, and vacuolated cytoplasm, often admixed with Touton-type giant cells. However, when xanthomatous cells and Touton-type giant cells are absent, it can be challenging to accurately diagnose JXG as the presentation can mimic malignant tumors.…”

Section: Discussionmentioning

confidence: 99%

“…17,18 To date, 38 cases of intraoral JXG have been reported. 14,15 Of those, seven cases involved adult patients, with ages varying between 35 and 64 years. 14,15 Clinically, three variants of oral JXG have been described, namely, the papular, nodular, and giant or macronodular forms.…”

Section: Discussionmentioning

confidence: 99%

“…14,15 Of those, seven cases involved adult patients, with ages varying between 35 and 64 years. 14,15 Clinically, three variants of oral JXG have been described, namely, the papular, nodular, and giant or macronodular forms. Notably, case studies on four children and two adults have described two histopathological variants (non-lipidized and mononuclear).…”

Section: Discussionmentioning

confidence: 99%

“…JXG is a histiocytic disorder with frequent cutaneous involvement 14–16 . Microscopically, JXG exhibits a proliferation of histiocytes with a foamy, eosinophilic, and vacuolated cytoplasm, often admixed with Touton‐type giant cells.…”

Section: Discussionmentioning

confidence: 99%

“…However, when xanthomatous cells and Touton‐type giant cells are absent, it can be challenging to accurately diagnose JXG as the presentation can mimic malignant tumors 17,18 . To date, 38 cases of intraoral JXG have been reported 14,15 . Of those, seven cases involved adult patients, with ages varying between 35 and 64 years 14,15 .…”

Section: Discussionmentioning

confidence: 99%

See 4 more Smart Citations

Solitary xanthoma affecting the oral mucosa: Immunohistochemical analysis and literature review

Silveira

Neto²,

Silva

et al. 2022

J Cutan Pathol

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 3 more Smart Citations

Solitary xanthoma affecting the oral mucosa: Immunohistochemical analysis and literature review

Silveira

Neto²,

Silva

et al. 2022

J Cutan Pathol

View full text Add to dashboard Cite

Frequent detection of genetic aberrations reveals novel pathogenesis and treatment modalities in systemic juvenile xanthogranuloma

Xu,

Ma,

Yao

et al. 2023

Pediatric Investigation

View full text Add to dashboard Cite

Frequent detection of genetic aberrations reveals novel pathogenesis and treatment modalities in systemic juvenile xanthogranulomaJuvenile xanthogranuloma (JXG), the most common form of non-Langerhans cell histiocytosis (non-LCH), is generally confined to the skin during infancy and early childhood. 1 JXG rarely involves extracutaneous tissues or systemic organs, resulting in high morbidity and mortality rates. Clonality in JXG has been verified by histopathological and genetic analyses, which have shown it to be tumorous rather than a reactive disorder of JXG. 2 However, the exact etiopathogenesis of JXG remains unclear.Diverse organs and systems can be involved in systemic JXG (SJXG). The most common extracutaneous sites include the eyes, central nervous system (CNS), liver, and lungs. Compared with cutaneous JXG, SJXG usually requires more aggressive treatment. Conventionally, the first-line treatment for SJXG is based on chemotherapy used in Langerhans cell histiocytosis (LCH), such as corticosteroids and vinblastine, with various responses. 3 Alternative regimens include methotrexate, cladribine, and cytarabine when initial chemotherapy fails.In the past decade, genomic studies have been conducted on histiocytosis, including JXG, and distinctive genetic abnormalities have been discovered (e.g., BRAFV600E mutations and ALK translocations). 4,5 These novel genetic discoveries further confirmed the tumorigenesis of JXG and highlighted the possibility of therapeutic targets in refractory or severe cases. GENETIC ABERRATIONS IN THE PATHOGENESIS OF SJXGJXG is involved in the clonal proliferation of histiocytes by human androgen receptor assay (HUMARA). 2 However, the drivers of cell proliferation in JXG remain to be elucidated. Several cases of JXG have been reported

show abstract

Juvenile and adult xanthogranuloma: A 30-year single-center experience and review of the disorder and its relationship to other histiocytoses

Salari

Dehner

2022

Annals of Diagnostic Pathology

View full text Add to dashboard Cite

Oral Juvenile Xanthogranuloma: A Clinicopathological, Immunohistochemical and BRAF V600E Study of Five New Cases, with Literature Review

Cited by 6 publications

References 36 publications

Solitary xanthoma affecting the oral mucosa: Immunohistochemical analysis and literature review

Solitary xanthoma affecting the oral mucosa: Immunohistochemical analysis and literature review

Frequent detection of genetic aberrations reveals novel pathogenesis and treatment modalities in systemic juvenile xanthogranuloma

Juvenile and adult xanthogranuloma: A 30-year single-center experience and review of the disorder and its relationship to other histiocytoses

Contact Info

Product

Resources

About